Iris hypoplasia in incontinentia pigmenti

This report describes a young girl with ocular and dermatologic manifestations of incontinentia pigmenti. Fortunately, she has thus far been spared the more severe systemic problems, as well as any vision-threatening complications. However, a unique finding of sector stromal hypoplasia of the iris in her left eye was noted. It is interesting to speculate that this iris pigmentary disturbance may in some way relate to the underlying pigmentary disturbance seen in the skin and in the retinal pigment epithelium.